Heyde's Syndrome
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Cease Report Adv Res Gastroentero Hepatol Volume 2 Issue 4 - January 2017 DOI: 10.19080/ARGH.2017.04.555592 Copyright © All rights are reserved by Deepankar Kumar Basak Heyde’s syndrome: Rarely heard and often missed Deepankar Kumar Basak1*, Richmond Ronald Gomes2 and Md Samsul Arfin3 1Specialist-Gastroenterology, Square Hospitals Ltd., Bhangladesh 2Assistant Professor, Internal Medicine, Ad-din Sakina Medical College & Hospital, Bhangladesh 3Gasroenterology, Square Hospitals Limited., Bhangladesh Submission: November 11, 2016; Published: January 19, 2017 *Corresponding author: Deepankar Kumar Basak, MBBS, FCPS (Medicine), Specialist-Gastroenterology, Square Hospital Ltd., Dhaka, Bangladesh, Tel: Email: Abstract Bleeding from the gastrointestinal tract is very common and important problem in clinical practice. There are lots of causes of GI bleeding, gastrointestinal bleeding in an elderly female patient who is also suffering from HCV related decompensated CLD with multiple myeloma. Heyde’sbut sometimes syndrome it is is very now difficult known to belocate gastrointestinal and treat gastrointestinal bleeding from bleeding. angiodysplasic Here we lesions discuss due Heyde’s to acquired syndrome, vWD-2A an secondaryimportant tocause aortic of stenosis, and the diagnosis is made by confirming the presence of those three things. For this, a wide range of investigations and treatment showingmodalities gastrointestinal are now available. bleeding One aftershould aortic therefore valve makereplacement. an aggressive Old age attempt and co-morbidities to localize the may bleeding create site.a hindrance Newer endoscopic in valve replacement technologies or resectionmay prove surgery. beneficial. Some Aortic newer valve treatment replacement options is claimed like hormonal to minimize and orthalidomide even stop thetherapy bleeding look in promising such patients. but they But stillhave there inadequate are a few evidence reports behind them. Here, we discuss this clinical problem, strategies and evidence, areas of uncertainty, available guidelines, and our conclusions about Heyde’s syndrome. Keywords: Heyde’s syndrome; gastrointestinal angiodysplasias; Gastrointestinal bleeding Introduction transfusions. We describe a case of Heyde’s syndrome with Angiodysplasia is the most common vascular lesion of the multiple angiodysplastic lesions throughout the colon, mostly at gastrointestinal tract, and this condition may be asymptomatic, the distal part and discuss the various challenges in the diagnosis or it may cause gastrointestinal (GI) bleeding [1]. The vessel and treatment of such patients. walls are thin, with little or no smooth muscle, and the vessels are ectatic and thin. It is a degenerative lesion of previously Case Report healthy blood vessels found most commonly in the cecum and Mrs. Chaina Chakraborty, 71 year old pleasant lady, known proximal ascending colon but bleeding from proximal intestinal to have multiple myeloma (MULTIPLE MYELOMA-IgG Kappa angiodysplasias, and nasal bleeding is also reported [2,3]. After Myeloma and Beta2 Microglobulin-4.2) chronic liver disease diverticulosis, it is the second leading cause of lower GI bleeding (HCV related) with Esophageal Varices (grade 2), duodenal in patients older than 60 years. An association between colonic ulcer and aortic stenosis with IHD was admitted to SHL angiodysplasia and aortic stenosis was described by Edward C gastroenterology department through ER with the complaints Heyde et al. [4]. It is caused by the induction of Von Willebrand disease type IIA (vWD-2A) by a depletion of Von Willebrand weakness for same duration. She had previous history of of passage of black tarry stool for last five days and generalized hospital admission for several times in the last three months stenosis. The existence of this syndrome was debated for a factor (vWF) in blood flowing through the narrowed valvular with these same complaints and received 8-10 units of blood and blood products over that period. Now her hemoglobin 5 gastrointestinal bleeding did actually resolve after aortic valve considerable period of time when finally it was shown that the gm/dl, Haematocrit 20.9%, TC 10.5 K/uL, Platelets 40K/uL, PT, aPTT was normal. Echocardiogram shows narrow LVOT credence to the existence of this syndrome [2,5]. The bleeding replacement in many such patients, thereby giving a definite with Dynamic subvalvular Aortic Stenosis Gr 78/28mmHg could be severe and the patient usually requires multiple blood Adv Res Gastroentero Hepatol 2(4): ARGH.MS.ID.555592 (2017) 0071 Advanced Research in Gastroenterology & Hepatology with no AR. Endoscopy shows grade 2 esophageal varix but no We do argon plasma coagulation (APC) of angiodysplastic active bleeding from varix. Colonoscopy shows angiodysplastic lesion in colon as far as possible in 2 sessions with injection of lesions through the colon with hamorrhoids.Bleeding occur octreotide. In this measure she was recovered well and there was most probably from angiodysplastic lesions but we don’t see any no bleeding at least 1 year follow-up. After that again melaena active bleeding from any angiodysplasia. Initially patient was started on/off. She was also treated by desmopressin nasal also platelet aphaeresis (Figure 1). measure decreases her incidence of melaena. We offer her to do managed with blood transfusion with fresh frozen plasma and spray with cap.danazol to increase vWF with inj octreotide. This aortic valvae replacement for further management but she was not interested. Discussion Angiodysplasia is the most common vascular lesion of the gastrointestinal tract, and this condition may be asymptomatic, or it may cause gastrointestinal (GI) bleeding [1]. Seventy- Figure 1: Angioplastic lesion in colon. seven percent of angiodysplasias are located in the cecum and ascending colon, 15% are located in the jejunum and ileum, and Our patient is an elderly lady with multiple co-morbidities. the remainder is distributed throughout the alimentary tract. She was treated by thalidomide for multiple myeloma that Nasal bleeding is also reported [2,3]. These lesions typically are was controlled. Two times EVL was done for HCV related nonpalpable and small (< 5 mm). Angiodysplasia may account decompensated CLD with feature of hypersplenism with low for approximately 6% of cases of lower GI bleeding. It may be platelet count. Her past history of multiple episodes of life- observed incidentally at colonoscopy in as many as 0.8% of threatening blood loss makes it necessary to do something to patients older than 50 years. The prevalence for upper GI lesions prevent gastrointestinal blood loss. The options available are is approximately 1-2%.Clinical presentation in patients with angiographic intervention, endoscopic intervention, intestinal resection, aortic valve replacement, and estrogen-progesterone therapy/thalidomide therapy. The patient herself along with angiodysplasia is usually characterized by maroon-colored stool, can be massive in approximately 15% of patients. In 20-25% of her relatives were unwilling for any major surgical intervention melena, or hematochezia. Bleeding is usually low grade, but it due to her advanced age and the multiple comorbidities that she anemia and stools that are intermittently positive for occult had, further compromising the likely outcome after the surgery. bleeding episodes, only tarry stools are passed. Iron deficiency blood can be the only manifestations of angiodysplasia in 10- There was a high chance of more angiodysplastic lesion in small 15% of patients. Bleeding stops spontaneously in greater than gut which is not approachable on colonoscopy and there was no 90% of cases but is often recurrent. An association between capsule endoscope in our country. colonic angiodysplasia and aortic stenosis was described by Angiographic intervention is also not preferable as there is Edward C Heyde et al. [4]. a possibility of existence of multiple unnoticed angiodysplasias, The exact mechanism of development of angiodysplasia possibility of gut infarction after angiography, and the deranged is not known, but chronic venous obstruction may play a role renal function of the patient would be a contraindication for [6,7]. Increased expression of angiogenic factors, like basic best and acceptable solution for this patient like aortic valve angiography. All these problems make it difficult to choose the factor (VEGF), is also believed to play a role in the pathogenesis replacement (Figure 2&3). fibroblast growth factor (bFGF) and vascular endothelial growth of colonic angiodysplasia [8]. In Heyde’s syndrome; it seems that bleeding occurs from pre-existing angiodysplasias in the gut. This bleeding is due to an acquired haematological defect caused by aortic stenosis [5,9]. Age-related senile tissue changes may predispose to angiodysplasia and the link between the angiodysplasia and aortic stenosis is controversial [10-12]. Various studies have described the acquired von Willebrand Figure 2: Argon plasma coagulation (APC). factor deficiency as being a reason for bleeding in Heyde’s syndrome [5,13]. The haematological defect is identified as Willebrand factor (vWF) [14]. The high shearing force caused deficiency of high molecular weight (HMW) multimers of von by blood jet in aortic stenosis uncoils the HMW multimers of vWF exposing the vWF cleavage site for ADAMST [13]. This leads to selective loss of HMW multimers of vWF due to increased Figure 3: EVL. Willebrand syndrome type 2A). There is an evidence showing proteolysis. Acquired